Co esponding au ho : Emmanuel N. Kibassim
Copy igh © 2025 Au ho (s) e ain he copy igh o his a icle. This a icle is published unde he e ms o he C ea i e Commons A ibu ion License 4.0.
Case epo : Le ca diac sympa he ic dene a ion ia ho aco omy in a newbo n wi h
congeni al long QT synd ome
Emmanuel N. Kibassim 1, *, Najeebullah Bina 1, Philippe Aca 2, Y es Dulac 2, Khaled Hadeed 2, Gé ald
Chausse ay 3, Julie Gobin 3 and Lionel Be homieu 3
1 Depa men o Ca dio ho acic Su ge y, Hôpi al des En an s-Pu pan, CHU Toulouse, F ance.
2 Depa men o Pedia ic Ca diology, Hôpi al des En an s-Pu pan, CHU Toulouse, F ance.
3 Depa men Pedia ic and Neona al Medical-Su gical In ensi e Ca e Uni , Hôpi al des En an s-Pu pan, CHU Toulouse,
F ance.
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 1983-1986
Publica ion his o y: Recei ed on 05 Ap il 2025; e ised on 11 May 2025; accep ed on 14 May 2025
A icle DOI: h ps://doi.o g/10.30574/wja .2025.26.2.1875
Abs ac
Objec i e: Congeni al Long QT Synd ome (LQTS) is a a e channelopa hy associa ed wi h malignan a hy hmias and
sudden ca diac dea h. This case epo aims o highligh he ole o Le Ca diac Sympa he ic Dene a ion (LCSD) as a
escue he apy in neona es wi h d ug- e ac o y LQTS.
Me hods: We p esen he case o a emale neona e diagnosed wi h congeni al LQTS ype 8 (Timo hy synd ome),
complica ed by 2:1 a io en icula block and ecu en o sades de poin es despi e pacemake implan a ion, be a-
blockade, mexile ine, and magnesium. Due o pe sis en a hy hmias and a ansien esponse o le s ella e ganglion
block, LCSD was pe o med ia ho aco omy.
Resul s: The su ge y in ol ed esec ion o he le s ella e ganglion and ho acic ganglia T2–T4 h ough a le
ho aco omy app oach. The immedia e pos ope a i e cou se was une en ul. Gene ic analysis con i med a CACNA1C
mu a ion. Despi e he in e en ion, he pa ien died on pos ope a i e day se en due o p og essi e ca diac ailu e.
Conclusion: This case unde sco es he po en ial ole o LCSD in se e e neona al LQTS un esponsi e o con en ional
he apy. Howe e , i s e icacy appea s o depend hea ily on gene ic sub ype and iming o in e en ion. Ea ly gene ic
es ing and imely su gical decision-making may imp o e ou comes in such high- isk pa ien s. Fu he in es iga ion is
needed o de e mine op imal indica ions and iming o LCSD in neona es wi h LQTS.
Keywo ds: Ca diac sympa he ic dene a ion; Congeni al a hy hmia; Long QT synd ome; Neona e; Pedia ic ca diac
su ge y
1. In oduc ion
Long QT Synd ome (LQTS) is a a e gene ic diso de o ca diac epola iza ion caused by mu a ions a ec ing ion
channels. This condi ion can lead o o sades de poin es and ca diac a es , wi h an es ima ed p e alence o 1 in 2,000
li e bi hs [1]. Diagnosis is based on elec oca diog aphic indings, no ably a p olonged co ec ed QT in e al (QTc),
and may be con i med by gene ic es ing. Fi s -line ea men consis s o be a-blocke s, wi h pacemake o implan able
ca dio e e -de ib illa o (ICD) he apy conside ed in mo e se e e cases. Le Ca diac Sympa he ic Dene a ion (LCSD),
by educing sympa he ic inpu o he myoca dium, ep esen s an al e na i e he apeu ic op ion in cases e ac o y o
con en ional ea men .
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 1983-1986
1984
2. Ma e ials and Me hods
A emale neona e was deli e ed a 37 weeks o ges a ion by cesa ean sec ion due o e al b adyca dia de ec ed as ea ly
as 23 weeks (2:1 a io en icula block). A bi h, she p esen ed wi h pe sis en b adyca dia (50–60 bpm), a co ec ed
QT in e al (QTc) > 600 ms, and 2:1 AV block. Elec oca diog aphy (ECG) con i med he diagnosis o congeni al Long
QT Synd ome (LQTS). In he absence o a amily his o y, a pacemake was implan ed on day 1. P op anolol was ini ia ed
a 4.7 mg/kg/day, and he in an was discha ged home on day 18.
On day 21, he pa ien expe ienced a ca diac a es due o o sades de poin es, which was success ully esusci a ed a e
20 minu es o ad anced li e suppo . Upon admission o he in ensi e ca e uni , o sades de poin es pe sis ed despi e
pacemake op imiza ion, ini ia ion o mexile ine, and in a enous magnesium sul a e. Echoca diog aphy e ealed
bi en icula dys unc ion seconda y o he ca diac a es , as well as a pe sis en duc us a e iosus.
A empo a y le s ella e ganglion block using Na opeine (2 mg/ml a 1 mg/kg) esul ed in ansien imp o emen ,
p omp ing he indica ion o su gical Le Ca diac Sympa he ic Dene a ion (LCSD).
Su gical P ocedu e: LCSD was pe o med ia a le ho aco omy unde gene al anes hesia. The in an was posi ioned in
a igh la e al decubi us posi ion, wi h he le a m abduc ed abo e he head and a oll placed unde he igh shoulde .
A pos e ola e al skin incision was made in he ou h le in e cos al space, be ween he mid-axilla y and pa a e eb al
lines. Muscle laye s (la issimus do si, se a us an e io , and in e cos al muscles) we e dissec ed using elec ocau e y.
The le lung was co e ed wi h mois gauze and gen ly e ac ed an e io ly and in e io ly using a so issue e ac o .
The pa ie al pleu a was ca e ully opened and exposed. The sympa he ic unk was i s iden i ied along he pos e io
aspec o he ho acic ca i y, whe e i was eadily isualized. The le s ella e ganglion, along wi h he T2 o T4 ho acic
ganglia, we e iden i ied, esec ed, and sen o his opa hological analysis.
The p ocedu e was une en ul, and he pa ien was ans e ed o he in ensi e ca e uni o pos ope a i e moni o ing.
Figu e 1 Exposu e o he le ho acic sympa he ic chain du ing pedia ic LCSD
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 1983-1986
1985
3. Discussion
His opa hological and gene ic analysis o he esec ed ganglia con i med he diagnosis o ype 8 Long QT Synd ome
(LQT8), also known as Timo hy synd ome—an ex emely a e o m (<1 in 1,000,000), associa ed wi h mul isys em
in ol emen and mu a ions in he CACNA1C gene [2]. The se e i y o his geno ype may explain he lack o esponse o
con en ional he apy.
LCSD is conside ed a las - eso ea men in d ug- e ac o y LQTS. While well-documen ed in olde child en and
adul s, i s use in neona es emains a e. The p ocedu e educes sympa he ic one, he eby lowe ing he isk o li e-
h ea ening a hy hmias. Se e al s udies ha e demons a ed ha LCSD signi ican ly educes a hy hmic episodes,
including ecu en syncope and he isk o sudden ca diac dea h [3]. Howe e , i s e icacy is geno ype-dependen and
a ies based on he ex en o which he au onomic ne ous sys em con ibu es o a hy hmia gene a ion [4].
A comp ehensi e li e a u e e iew, including he a icle by Du hoi i led "Sympa he ic dene a ion in he managemen
o en icula achyca dia" [5], highligh s he ole o sympa hec omy in con olling e ac o y a hy hmias. This s udy
suppo s he concep ha sympa he ic modula ion ia dene a ion may p o ide he apeu ic bene i in selec ed pa ien s
wi h d ug- esis an en icula hy hm diso de s. Al hough mos da a come om adul and olde pedia ic popula ions,
such an app oach could be conside ed mo e sys ema ically in neona es wi h se e e o ms o LQTS.
An al e na i e o he ho aco omy app oach has been p oposed by Ode o e al. in hei a icle "Le ca diac sympa he ic
dene a ion o he p e en ion o li e- h ea ening a hy hmias: The su gical sup acla icula app oach o ce ico ho acic
sympa hec omy." This echnique uses a sup acla icula app oach o access and ansec he sympa he ic chain, making
he p ocedu e less in asi e and a oiding ho aco omy- ela ed complica ions [6]. Al hough p omising, his app oach
emains unde s udied in neona al LQTS, and u he esea ch is equi ed o assess i s long- e m e icacy and sa e y.
In ou case, he decision o p oceed wi h LCSD, al hough delayed, was jus i ied by he pe sis ence o a hy hmias despi e
maximal he apy. Un o una ely, he clinical ou come was un a o able: he pa ien died se en days pos ope a i ely.
This case aises se e al impo an ques ions:
• Would ea lie LCSD ha e imp o ed he ou come?
• Could a neona al ICD implan a ion ha e been easible?
• Would geno ype-guided he apy ha e al e ed he disease cou se?
Ab é ia ions
• LQTS : Long QT in e al Synd omes
• LCSD : Le Ca diac Sympa he ic Dene a ion
• ICD : Implan able Ca dio e e -De ib illa o
• AV BLOCK : A ioVen icula Block
• ECG : Elec oCa dioG aphy
• LQT8 : Type 8 o Long QT Synd ome
• QTc : Co ec ed QT in e al
• HRS : Hea Rhy hm Socie y
• EHRA : Eu opean Hea Rhy hm Associa ion
• APHRS : Asia Paci ic Hea Rhy hm Socie y
4. Conclusion
Se e e congeni al Long QT Synd ome is a pedia ic eme gency ha equi es specialized managemen . LCSD may
ep esen a he apeu ic op ion in e ac o y cases; howe e , i s e icacy is highly dependen on he pa ien 's geno ype
and he iming o he in e en ion. This case unde sco es he impo ance o ea ly gene ic es ing and he need o
ailo ed he apeu ic s a egies in se e e neona al p esen a ions.
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 1983-1986
1986
Compliance wi h e hical s anda ds
Disclosu e o con lic o in e es
The au ho s decla e no con lic s o in e es .
S a emen o e hical app o al
This publica ion complies wi h con iden iali y s anda ds. E hics commi ee app o al was no equi ed o his
anonymous clinical case epo .
S a emen o in o med consen
W i en in o med consen was ob ained om he pa ien ’s pa en s o he publica ion o his case.
Au ho Con ibu ions:
All au ho s had ull access o he da a and con ibu ed equally o he analysis and w i ing o he manusc ip . All au ho s
app o ed he inal e sion and a e accoun able o all aspec s o he wo k.
Re e ences
[1] Schwa z PJ, e al. P e alence o he congeni al long-QT synd ome. Ci cula ion. 2009;120:1761-1767.
[2] O phane . Synd ome de Timo hy. h ps://www.o pha.ne /conso /cgibin/OC_Exp.php?lng=FR&Expe =65283.
Accessed Feb ua y 21, 2025.
[3] P io i SG, Wilde AA, Ho ie M, e al. Execu i e summa y: HRS/EHRA/APHRS Expe Consensus S a emen on he
Diagnosis and Managemen o Pa ien s wi h Inhe i ed P ima y A hy hmia Synd omes. Hea Rhy hm Socie y.
2015;12(10):e237-e268.
[4] Vincen GM, e al. Spec um o symp oms and QT in e als in long QT synd ome. N Engl J Med. 1992;327(12):846-
852.
[5] Du hoi . Déne a ion sympa hique dans la p ise en cha ge des achyca dies en iculai es. Ca diologie P a ique.
2024.
[6] Ode o A, e al. Le ca diac sympa he ic dene a ion o long QT synd ome. Hea Rhy hm. 2009;6(6):752-759.
